SESSION TYPE: Cardiovascular Cases
PRESENTED ON: Wednesday, October 24, 2012 at 11:15 AM - 12:30 PM
INTRODUCTION: Primary cardiac lymphoma (PCL) is a localized extranodal lymphoma of the heart without extracardiac involvement . It is a rare entity representing 1.3% of primary cardiac tumors and 0.5% of extranodal lymphomas . Presenting symptoms are nonspecific and often delay diagnosis.
CASE PRESENTATION: A 63 year-old male presented with symptoms of heart failure and Intermittent sinus pauses of four seconds on electrocardiography. Transthoracic echocardiogram identified a 4 cm x 2 cm right atrial (RA) mass thought to be thrombus. The patient was started on anticoagulation therapy and discharged. Thirteen days later, the patient returned with frequent near syncopal episodes and 10 second asystolic pauses on electrocardiography. Cardiac magnetic resonance imaging demonstrated a lobulated RA mass. Surgical exploration revealed tumor seedings at the roof of the left atrium. The tumor infiltrated the RA free wall from the superior vena cava (SVC)-RA junction extending down to the right pulmonary veins. The mass was debulked and the SVC inlet was liberated from the infiltrative tissue. Immunohistochemistry findings identified the tissue as a non-Hodgkin small lymphocytic lymphoma of B-cell immunophenotype. Whole body positron emission tomography—computed tomography scan and a bone marrow biopsy ruled out extracardiac involvement; a diagnosis of PCL was confirmed. Two years later, the patient remains free of symptoms.
DISCUSSION: Primary cardiac lymphoma is a rare and elusive diagnosis. Nonspecific symptoms and a low index of suspicion delay diagnosis and contribute to the associated poor prognosis of PCL (3). In our experience, worsening symptoms of sinus dysfunction and persistent RA mass led to further workup and early diagnosis of PCL. Indeed, the presence of dysrhythmia has been associated with improved overall survival in patients with PCL . Other factors that may have contributed to the good outcome in our patient include an immunocompetent state and lack of extracardiac and left ventricular involvement 
CONCLUSIONS: Primary cardiac lymphoma should be in the early differential of refractory intracardiac thrombus and arrhythmia.
1) McAllister HA, F.J., Atlas of Tumor Pathology. Tumors of the cardiovascular system. Vol. Fasicle 15. 1978, Washington, DC: Armed Forces Institute of Pathology
2) Rockwell, L., et al., Cardiac involvement in malignancies. Case 3. Primary cardiac lymphoma. Journal of clinical oncology : official journal of the American Society of Clinical Oncology, 2004.
3) Petrich, A., S.I. Cho, and H. Billett, Primary cardiac lymphoma: an analysis of presentation, treatment, and outcome patterns. Cancer, 2011. 117(3): p. 581-9.
DISCLOSURE: The following authors have nothing to disclose: Mohamed Algahim, Moritz Wyler Von Ballmoos, Ghulam Murtaza, Karl Takabayashi, Hossein Almassi
No Product/Research Disclosure InformationMedical College of Wisconsin, Wawatosa, WI